Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023467 (acute myeloid leukemia)
 35,200 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency and clinical significance of the pseudo-Chediak-Higashi (PCH) anomaly were studied in 20 children with acute myeloid leukemia (AML) M2 in the FAB nomenclature. PCH granules were recognized as giant eosinophilic granules, measuring up to 5 microns, in the cytoplasm of leukemic cells on smears. At the electron microscope level, most PCH granules were round to oval and outlined by a limiting membrane, and contained homogeneous, granular, crystalloid, rod-like or myelin-like materials. The PCH anomaly was demonstrable in five (25.0%) of the 20 patients, which indicates that the anomaly is not rare in childhood AML M2. There were no differences between PCH anomaly-positive and PCH anomaly-negative groups with regard to hepatosplenomegaly, hemoglobin levels, white blood cell counts, bone marrow cellularity, t(8q-, 21q+) chromosome abnormalities or prognoses. Circulating leukemic cells were observed less frequently in the PCH anomaly-positive group than in the PCH anomaly-negative group (p less than 0.05); the leukemic cells were not demonstrable in three of the five patients in the former group, although they were detected in all 15 patients in the latter group. The existence of PCH granules and/or a defect of the cytoskeleton responsible for the PCH anomaly in leukemic cells may impede their movement from the bone marrow to the peripheral blood.
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PMID:Pseudo-Chediak-Higashi anomaly in acute myeloid leukemia (M2) of childhood. 208 66

This report describes three cases with Down's syndrome. These cases initially had transient abnormal myelopoiesis (TAM), from which they recovered spontaneously. They finally developed into overt acute leukemia characterized by an increase of blasts, hepatosplenomegaly, and elevated lactic dehydrogenase. Of these three cases, one was thought to have ANLL, which broke out 5 months after spontaneous remission. The other two had ALL, each occurring 8 and 9 years later. Chromosomal abnormality, in addition to trisomy 21, was detected in blast cells from one of the patients with acute leukemia. All three patients with acute leukemia experienced complete remission. However, two of the three patients relapsed and died. It is noted in the literature that remission is permanent in most cases of TAM, and is rarely terminated by leukemic relapse. In view of our observations, the importance of following up on such patients who evidence apparent remission of their leukemia-like disorder is emphasized.
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PMID:[Transient abnormal myelopoiesis followed by acute leukemia in children with Down syndrome]. 215 Apr 19

Bone marrow monosomy 7 is the most frequent karyotypic abnormality found in patients with chronic myeloproliferative disorders. To a review of 46 previously reported pediatric patients we add three additional cases. Clinical presentation is usually dependent upon which cell lines are most perturbed in this pluripotent stem cell disorder. Sixteen (35%) children presented by their first birthday and 35 (76%) by their sixth birthday. Distinctive differences in presentation exist between infants, children, and adolescents. Younger patients were more symptomatic and had greater degree of hepatosplenomegaly and leukocytosis. The prognosis is very poor and death usually occurs within two years from complications attributable to cytopenias, cellular dysfunction, or transformation to acute nonlymphocytic leukemia. Implications for therapy are discussed.
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PMID:Monosomy 7 syndrome. Clinical heterogeneity in children and adolescents. 229 85

We report the clinical and pathologic findings in one case of mast cell leukemia observed in a series of 60 patients with systemic mast cell disease. The leukemic variant of systemic mast cell disease is rapidly fatal (mean duration of survival, less than 6 months) in contrast to most nonleukemic cases, which follow an indolent clinical course. On the basis of our case and eight previously reported cases, mast cell leukemia is characterized by a substantial increase in atypical mast cells in the peripheral blood, diffuse infiltration with atypical mast cells in the bone marrow, a strong association with peptic ulcer disease, prominent constitutional symptoms, and hepatosplenomegaly. These cases should be distinguished from malignant mastocytosis without a substantial number of circulating atypical mast cells and also cases of acute nonlymphocytic leukemia that arise in the background of systemic mast cell disease.
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PMID:Mast cell leukemia: report of a case and review of the literature. 309 98

A case of lepromatous leprosy with erythema nodosum leprosum (ENL) presenting as a myeloid leukemoid reaction is reported. Very high leucocyte count with immaturity of the cells in myeloid series was present in peripheral blood. High leucocyte alkaline phosphatase score, absence of hepatosplenomegaly and transient nature of leukemoid reaction differentiated it from chronic myeloid leukemia and acute myeloblastic leukemia. The possible mechanisms of leukemoid reaction in ENL are discussed.
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PMID:Leukemoid reaction in erythema nodosum leprosum in a leprosy patient. 325 36

A new translocation t(1;11;4)(1pter----1p32::11q23----11q13::4p16--- -4qter) was found in the peripheral blood of a patient with congenital acute myeloblastic leukemia (AML). It was concluded that this translocation may represent a new mutation, which caused the leukemia with very high leukocytosis, hepatosplenomegaly, leukemic infiltration of the majority of the organs, and a very poor prognosis.
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PMID:A new translocation t(1;4;11) in congenital acute nonlymphocytic leukemia (acute myeloblastic leukemia). 347 1

We determined the clinical and biological correlates of coagulopathy in a large series of patients with untreated childhood acute leukemia. Twenty-five of 805 children with acute lymphoblastic leukemia (ALL) (3.1%) and 27 of 195 with acute myeloid leukemia (AML) (13.8%) met any two of three requirements for a coagulation disorder: fibrinogen level less than 150 mg/dL; fibrin degradation products greater than 10 micrograms/mL; and prolongation of prothrombin time (PT) greater than 12 seconds, activated partial thromboplastin time (PTT) greater than 45 seconds, or thrombin time (TT) greater than 18 seconds. Patients with ALL complicated by abnormal coagulation were more likely to be boys and to have a T-cell immunophenotype, a high leukocyte count, a mediastinal mass, leukemic involvement of the CNS, hepatosplenomegaly, and L2 blast cell morphology. These features were highly interrelated, with only T-cell markers and CNS involvement achieving independent significance in a multivariate logistic regression model. Hepatomegaly, blast cell morphological subtype (French-American-British [FAB] M3, M4, and M5) and age less than 2 years were each associated with coagulopathy in patients with AML, although age failed to retain importance after logistic regression analysis. The presence of coagulopathy at diagnosis of ALL did not influence the rate of remission induction (P = .55). By contrast, only 14 of 27 children with coagulopathy at diagnosis of AML achieved a complete remission (CR), compared with 129 of 168 other patients who lacked this complication (P = .003). After multivariate analysis, coagulopathy remained independently associated with failure to attain remission in AML (P = .02). Fatal hemorrhagic complications arising in the CNS or lungs accounted for nine of the 13 induction failures in this group. The presence or absence of coagulopathy had no discernible influence on treatment outcome among patients with either ALL or AML who attained a CR. Laboratory evidence of a coagulation defect may be useful in identifying patients with AML who have a greater risk of induction failure and, hence, require close surveillance and intensive replacement therapy to prevent fatal hemorrhagic complications.
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PMID:The clinical and biological correlates of coagulopathy in children with acute leukemia. 352 66

The present study was undertaken to investigate the hemopoietic cell from which malignant change evolves in juvenile dyshemopoiesis with monosomy 7. Two male patients, aged 18 and 5 months, were studied using progenitor assays combined with cytogenetics. Both had hepatosplenomegaly, cytopenias and a cellular marrow. The karyotype in direct marrow was 45,XY-7/47,XY,+8/46,XY in patient 1 and 45,XY,-7/46,XY in patient 2. Patient 1 received chemotherapy but developed acute nonlymphocytic leukemia after 17 months and died 20 months after diagnosis. During this time marrow metaphases with 45,XY,-7 increased to 100% (25/25). Patient 2 received an allogeneic marrow transplant 4 months after diagnosis which did not engraft. In both patients progenitors of both small (CFU-E) and large (BFU-E) erythroid colonies were present at normal frequencies. However, the colonies produced were small and poorly hemoglobinized with some erythropoietin-independent maturation. Progenitors of large granulocyte/macrophage colonies (CFU-GM) were present at an elevated frequency in the marrow of patient 1 and in the blood all progenitor classes were markedly increased. Cytogenetic analysis of colonies from this patient showed BFU-E to be 45,XY,-7 or 47,XY,+8 and CFU-GM to be 45,XY,-7 or 47,XY,+8 or 46,XY. In patient 2, most BFU-E were 45,XY,-7, although a few were 46,XY. These data indicate that malignant change in this disease involves hemopoietic stem cells capable of erythroid and in at least some cases, myeloid differentiation.
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PMID:Juvenile monosomy 7 syndrome: evidence that the disease originates in a pluripotent hemopoietic stem cell. 362 12

Within a few years of the introduction of effective systemic chemotherapy for the treatment of acute leukemia, central nervous system (CNS) leukemia emerged as a significant problem. The strategy for treatment of leukemia is that one achieves cure by destruction of all leukemic cells, through this cannot be verified with present technology. The incidence of CNS-leukemia and the factors influencing this complication have been studied in a group of 131 cases with acute childhood leukemia. The overall incidence of CNS-leukemia was 30% in acute lymphocytic leukemia (ALL), 8% in acute myeloblastic leukemia (AML). However, cumulative incidence of CNS-leukemia calculated by modified Life-Table Method showed 56.8% in children who survived over 48 months after diagnosis of ALL. It was considered that the chief cause of the increased incidence of this complication was prolongation of survival. The CNS-leukemia was significantly greater in patients with under 10 year of age (p greater than 0.05), high initial leukemic cell counts (p less than 0.025), high appearance rate of leukemic cells in peripheral blood (over 50%) (p less than 0.005), and hepatosplenomegaly, especially lymph node enlargement (p less than 0.05). Blood-borne metastasis was considered to be probably the chief route of leukemic infiltration to the CNS. Between 1972 and 1978, 153 children with ALL were treated with multiple methods of CNS-prophylaxis, and were analyzed in relation to treatment regimens, age, sex and initial hematologic status. Patients received CNS-prophylaxis; Group I: three doses of intrathecal methotrexate (MTX 12 mg/m2) and hydrocortisone (HDC 12 mg/m2), Group II: same as in Group I followed by cyclic MTX and HDC, Group III: same as in Group I plus 2,400 rads of cranial irradiation. CNS-leukemia terminated complete remission 25 of 153 patients (16.3%). The cumulative incidence of CNS-leukemia at 4 year calculated by the Kaplan-Meier Method was 40.5% in Group I, 26.9% in Group II, and 14.5% in Group III. Development of CNS-leukemia was more frequent in male than in female (p less than 0.05), and in patients with initial high leukocyte counts (WBC greater than 25,000/mm3). We conclude that the combination of cranial irradiation and intrathecal MTX and HDC was highly efficacious. However, more intensive CNS-prophylaxis should be needed for such a high risk patients in male with high initial WBC.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Acute leukemia]. 647 26

40 children (23 males, 17 females) have been diagnosed have ANLL during the period from february 1970 to september 1981. According to FAB classification, 24 cases were M1,-M2, 9 M3, 3 M4, 3 M5 and 1 M6. At diagnosis, 20 patients (50%) had leukocytes less than 10.000/mmc, 6 (15%) had leukocytes greater than 50.000mmc. Hb levels was 7 g% in 16 patients (40%); 10 children had hepatosplenomegaly (25%), 7 splenomegaly (18%) and 5 lymphoadenomegaly (13%). 4 patients had cutaneous or mucous infiltrates. None had meningeal involvement at diagnosis. According to the year of diagnosis, 3 groups can be identified. In the group I (1970-73), 11 patients have been treated with not codified combination chemotherapy as ARA-C, 6-TG, DNR, CTX, Metil-GAG. In the group II (1974-76) and in the group III (1977-81), the patients (respectively 12 and 17) have been treated according to the following protocols: LAM-5 (3), TRAP (5), COAP (1), LAM 80 (2), AIL 7402 (8), AIL 7604, AIL 7801 (6). Immunotherapy has been performed in 7 cases. CNS prophylaxis (MTX i.t. +/-ARA-C +/- RT) was given in 5 patients of group II and in 6 of group III. I patients of group I (45%), 6 of group II (50%) and 13 of group III (76%) achieved CR. Median duration of remission was 5 months in the group I and in 17 in group II and III.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute non-lymphatic leukemia in children]. 654 20


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